Bickle Kelly, Roark Tom R, Hsu Sylvia
University of South Florida, College of Medicine, Tampa, USA.
Am Fam Physician. 2002 May 1;65(9):1861-70.
Bullous dermatoses can be debilitating and possibly fatal. A selection of autoimmune blistering diseases, including pemphigus vulgaris, paraneoplastic pemphigus, bullous pemphigoid, cicatricial pemphigoid, dermatitis herpetiformis and linear IgA dermatosis are reviewed. Pemphigus vulgaris usually starts in the oral mucosa followed by blistering of the skin, which is often painful. Paraneoplastic pemphigus is associated with neoplasms, most commonly of lymphoid tissue, but also Waldenström's macroglobulinemia, sarcomas, thymomas and Castleman's disease. Bullous pemphigoid is characterized by large, tense bullae, but may begin as an urticarial eruption. Cicatricial (scarring) pemphigoid presents with severe, erosive lesions of the mucous membranes with skin involvement in one third of patients focused around the head and upper trunk. Dermatitis herpetiformis is intensely pruritic and chronic, characterized by papulovesicles and urticarial wheals on the extensor surfaces in a grouped or herpetiform, symmetric distribution. Linear IgA dermatosis is clinically similar to dermatitis herpetiformis, but it is not associated with gluten-sensitive enteropathy as is dermatitis herpetiformis.
大疱性皮肤病可能使人衰弱，甚至可能致命。本文综述了一系列自身免疫性大疱性疾病，包括寻常型天疱疮、副肿瘤性天疱疮、大疱性类天疱疮、瘢痕性类天疱疮、疱疹样皮炎和线状IgA大疱性皮病。寻常型天疱疮通常始于口腔黏膜，随后皮肤出现水疱，常伴有疼痛。副肿瘤性天疱疮与肿瘤有关，最常见于淋巴组织肿瘤，但也可见于华氏巨球蛋白血症、肉瘤、胸腺瘤和卡斯尔曼病。大疱性类天疱疮的特征是出现大的、紧张性水疱，但也可能开始表现为荨麻疹样皮疹。瘢痕性（瘢痕性）类天疱疮表现为严重的黏膜糜烂性病变，三分之一的患者有皮肤受累，主要集中在头部和上躯干周围。疱疹样皮炎剧烈瘙痒且呈慢性，特征为在伸侧表面出现丘疹水疱和荨麻疹样风团，呈群集性或疱疹样、对称分布。线状IgA大疱性皮病在临床上与疱疹样皮炎相似，但不像疱疹样皮炎那样与麸质敏感性肠病相关。
